SPECIAL ISSUE ARTICLE

Life-Threatening Headaches in
Children: Clinical Approach and
Therapeutic Options
Nagma Dalvi, MD; and Lalitha Sivaswamy, MD

ABSTRACT of headache may occur. Table 1 pro-

vides an overview of symptoms, signs,
Life-threatening headaches in children can present in an apoplectic manner that gar- and appropriate imaging modalities that
ners immediate medical attention, or in an insidious, more dangerous form that may go can lead to a definitive diagnosis.
unnoticed for a relatively long period of time. The recognition of certain clinical charac-
teristics that accompany the headache should prompt recognition and referral to an insti- THUNDERCLAP HEADACHE
tution equipped with neuroimaging facilities, pediatric neurosurgeons, and neurologists. The term “thunderclap headache”
Thunderclap headaches, which reach a peak within a very short period of time, may be the (TCH) is applied to a severe, unanticipat-
presenting feature of conditions such as arterial dissection, venous sinus thrombosis, and ed headache that quickly (usually within
reversible cerebral vasoconstriction syndrome, which can be addressed by specific phar- a minute) reaches a crescendo. Adults
macological options instituted in an intensive care setting. On the other hand, subacute often describe it as “the worst headache
to chronic headaches that are accompanied by focal neurological signs, such as abducens of my life.” TCH was initially used in
nerve palsy, restriction of upward gaze, or papilledema, may be indicative of the need for reference to the pain of an unruptured
urgent imaging and neurosurgical referral. [Pediatr Ann. 2018;47(2):e74-e80.] cerebral aneurysm or the pain associated
with subarachnoid hemorrhage. None-

A
s most children who present to them in an expeditious manner may have theless, several disease states have been
a physician with headache have devastating consequences for the child recognized as presenting with TCH.1
primary headache disorders (ie, and carry a heavy medico-legal burden These include (1) leaking intracranial
disorders with no underlying identifiable for the pediatrician. aneurysm “sentinel headache”; (2) pitu-
organic brain disease) such as migraine The first half of this review focuses itary apoplexy; (3) arterial dissection;
and tension-type headache, life-threat- on headaches that present in a sudden (4) reversible cerebral vasoconstriction
ening headaches may go unnoticed by a manner, and the second half examines syndrome; (5) posterior reversible en-
busy clinician. In this article, we outline headaches that are of a subacute nature cephalopathy; (6) venous sinus thrombo-
some causes of headache that require and caused by raised intracranial pres- sis; (7) hypertensive crisis; and (8) spon-
rapid recognition, as failure to identify sure. Some overlap in the characteristics taneous intracranial hypotension
Causes of TCH that are commonly
Nagma Dalvi, MD, is a Child Neurologist and Neuro-Oncologist, Nemours AI Dupont Children’s Hos- encountered in the pediatric age group
pital. Lalitha Sivaswamy, MD, is an Associate Professor of Pediatrics and Neurology, Wayne State Univer- are addressed in the following text.
sity School of Medicine, Children’s Hospital of Michigan.
Address correspondence to Lalitha Sivaswamy, MD, Wayne State University School of Medicine, Chil- Cervical Artery Dissection
dren’s Hospital of Michigan, 3950 Beaubien Street, Detroit, MI 48201; email: lsivaswamy@med.wayne. Dissection is caused by extravasa-
edu. tion of blood into the wall of the artery,
Disclosure: The authors have no relevant financial relationships to disclose. leading to occlusion of the vessel distal
doi:10.3928/19382359-20180129-04
to the site of pathology, which is a com-

e74 Copyright © SLACK Incorporated

 SPECIAL ISSUE ARTICLE

TABLE 1.

Thunderclap Headache: Etiologies, Investigations, and Interventions

Red Flag/Symptom/Sign Potential Etiology Imaging Method of Choice Intervention
Neck pain/tenderness Chiari malformation MRI and MRA of the brain and neck Surgical referral
Carotid bruit Anti-coagulation
Anti-platelet
Horner’s syndrome Carotid dissection MRA of the neck and brain Anti-coagulation
Anti-platelets
a
Positional headache Intracranial hypotension MRI of the brain with contrast MRI brain with contrast
(worse on sitting/standing) MRV Anti-coagulation
Venous sinus thrombosis
(worse while recumbent)
Thunderclap headache Several causes CT, MRI, MRA, MRV Dependent on etiology
Papilledema or any cranial Raised intracranial pressure CT, MRI Surgical referral
nerve involvement (eg, space-occupying lesion Spinal tap for opening pressure Acetazolamide
or IIH)
Occipital headache PRES MRI of the brain Control of hypertension
Chiari malformation Surgical intervention
Space-occupying lesions Reduce vasogenic edema (eg, mannitol)
Altered mental status (eg, Encephalitis EEG Antibiotics/antivirals
confusion, drowsiness or RCVS Spinal fluid analysis Immunemodulators (autoimmune encephalitis)
agitation) MRI Calcium channel blockers
Fever, skin rash Infection CSF analysis Antibiotics, antivirals, supportive treatment
a
Intracranial hypotension is characterized by low opening pressure on a spinal tap.
Abbreviations: CSF, cerebrospinal fluid; CT, computed tomography; EEG, electroencephalogram; IIH, idiopathic intracranial hypertension; MRA, magnetic resonance angiography; MRI, magnetic reso-
nance imaging; MRV, magnetic resonance venography; PRES, posterior reversible encephalopathy; RCVS, reversible cerebral vasoconstriction syndrome.

mon cause of stroke in children.2-4 Dis-
section of the carotid or vertebral arteries
classically presents with pain at the site
of pathology (ie, the affected side of the
neck), but may also cause referred pain
to the region of the ipsilateral orbit, jaw,
and ear. It is estimated that up to 25% of
children with dissection present with an
explosive headache that is of a thunder-
clap nature.5 The child may complain of
painful pulsatile tinnitus, loss of vision in
the affected eye, focal limb weakness on
the side opposite to the dissection, tongue
weakness, and altered taste. On physical
examination, Horner’s syndrome (ptosis,
miosis, and lack of sweating on the af-
fected side) and a bruit over the dissected
vessel are classic signs that can guide the
physician to a prompt diagnosis.
Although in most instances an event
such as a fall, roller-coaster ride, or un-
usual stretching of the neck may pre-
cede the dissection, several examples of
spontaneous dissection or dissection fol-
lowing a bout of coughing or sneezing
have been noted.6,7 Interestingly, young
adults with migraine are more prone to
developing dissection.8
Children with suspected dissection
should be referred immediately to the
emergency department of a hospital
with resources to perform appropriate
imaging studies. Diagnosis is estab-
lished by magnetic resonance angiog-
raphy (MRA) of the neck that shows
tapering of the vessel at the site of the
dissection (ie, “string sign” or “flame
sign” (Figure 1). Magnetic resonance
imaging (MRI) may also reveal stroke
in the region of the vascular territory.
Patients with confirmed dissection
should be admitted for inpatient evalu-
ation, neurological consultation, and, in
some cases, neurosurgical consultation.
Antiplatelet or anticoagulant therapy is
commonly employed in cases of dissec-
tion to prevent further occlusion of the
affected vessel.
Venous Sinus Thrombosis
Headache is the most common pre-
sentation of venous sinus thrombo-
sis and occurs in more than 75% of
cases.8Although in most instances the
pain tends to be insidious and occurs
over days to weeks, in about 10% of chil-
dren the pain is of explosive onset.9 The

PEDIATRIC ANNALS • Vol. 47, No. 2, 2018 e75


Figure 1. Magnetic resonance angiogram of the
brain demonstrating dissection leading to “flame
sign” of the carotid vessel and lack of blood flow
distally.
pain worsens with recumbency and with
coughing or sneezing. In addition, there
are usually symptoms and signs of raised
intracranial pressure, such as vomiting,
diplopia, and papilledema. Seizures and
stroke-like symptoms occur in up to 50%
of children.10 The manifestations of ve-
nous thrombosis can be highly varied
and mimic that of a brain tumor, stroke,
or infection. Children have a particular
predilection to venous thrombosis, espe-
cially when dehydrated or in the setting
of cancer, sepsis, meningitis, hyperco-
agulable states, and estrogen therapy.11
Over-reliance on computed tomogra-
phy scans of the brain may miss the di-
agnosis; therefore, imaging of the veins
using magnetic resonance venography
(MRV) is preferred. Treatment includes
a multidisciplinary approach with in-
volvement of a hematologist, neurolo-
gist, and inpatient admission for antico-
agulant therapy.
Reversible Cerebral
Vasoconstriction Syndrome
Reversible cerebral vasoconstric-
tion syndrome (RCVS) is a condition
in which there is transient dysregulation
of cerebral vascular tone leading to ar-
eas of constriction and microdilatation.
RCVS may be a primary entity, or as be-
ing increasingly recognized, it may be
secondary to a variety of factors. Illicit
drugs such as cocaine, sympathomimet-
ics such as pseudoephedrine (a frequent
e76
SPECIAL ISSUE ARTICLE
component of over-the-counter cough
and cold preparations), selective sero-
tonin reuptake inhibitors, diet pills, oral
contraceptives, and binge drinking can
be predisposing substances, as can the
postpartum state.12 The classic clinical
manifestation is an abrupt onset of re-
current headaches that are brief yet ex-
tremely painful, occurring for a couple
of weeks prior to presentation, some-
times associated with hypertension.13
Often, the headache is accompanied by
an altered level of consciousness and/
or focal neurological symptoms such
as seizures, motor weakness, loss of vi-
sion, or aphasia. RCVS can sometimes
overlap with another clinic-radiological
syndrome referred to as posterior revers-
ible encephalopathy syndrome (PRES),
which usually occurs in children with
uncontrolled hypertension.
In addition to classic features of
“strings and beads” on angiography or
MRA, imaging may also reveal stroke or
cerebral edema (Figure 2).
RCVS should be recognized as a
medical emergency that requires imme-
diate admission to an intensive care unit
and withdrawal of trigger factors. Initia-
tion of calcium channel blockers or mag-
nesium has been shown to be effective
in many instances. Over 90% of patients
do well, with complete reversal of neu-
rological manifestations when managed
appropriately;14 however some children
may develop stroke or hemorrhage and
suffer long-term consequences.
Primary TCH
A diagnosis of primary TCH can
only be made after other etiologies have
been excluded by appropriate investiga-
tions, including MRI, MRA, and MRV.
Normal cerebrospinal fluid (CSF) lev-
els must also be established before one
can label a headache as primary TCH.
Primary TCH can occur after cough,
sexual activity, or physical exertion, but
the same meticulous evaluation as dis-
cussed earlier must be completed before
one can diagnose a pediatric patient with
these entities.
INCREASED INTRACRANIAL
PRESSURE
Idiopathic Intracranial Hypertension
Idiopathic intracranial hypertension
(IIH), otherwise referred to as pseudutu-
mor cerebri, is a condition characterized
by elevated intracranial pressure without
ventriculomegaly or evidence of a mass
lesion on imaging. Fortunately, the term
“benign” intracranial hypertension has
been abandoned as it is now recognized
that IIH may cause serious consequences
in the form of irreversible vision loss. In
postpubertal children, obese girls tend to
be disproportionally affected, whereas in
young children there is equal predisposi-
tion in both boys and girls.15,16 Although
the term “idiopathic” is used (because in
most instances there is no clear-cut pre-
disposing factor), secondary causes such
as use of minocycline, vitamin A, and
oral retinoids should be actively sought.
Classic symptoms include daily
headache that is of a constant, nonpul-
satile nature and aggravated by cough-
ing or straining, pulsatile tinnitus (ie,
“whooshing” sound in the ear), diplopia
due to sixth cranial nerve palsy, and a
unique phenomenon referred to as tran-
sient visual obscurations. The latter oc-
curs in more than 75% of patients, is
characterized by loss of vision for less
than 60 seconds at a time, and may affect
one or both eyes.17 The most character-
istic feature on physical examination is
the presence of papilledema, which need
not be bilateral.
Evaluation includes urgent brain im-
aging with MRI and MRV and referral
to a neurologist and, in many instances,
an ophthalmologist for dilated fundus
examination. The absence of space-
occupying lesions or enlarged ventricles
Copyright © SLACK Incorporated

Figure 2. Magnetic resonance angiogram of a
child with reversible cerebral vasoconstriction
syndrome showing beaded appearance of the
posterior cerebral arteries.
favors the diagnosis of IIH, although an
astute radiologist may identify certain
positive features on imaging (eg, flatten-
ing of the globe, which is indicative of
papilledema or transverse sinus steno-
sis) that alert the clinician to the correct
diagnosis (Figure 3). Confirmation of
the diagnosis requires measurement of
opening pressure during a lumbar punc-
ture. Values of greater than 28 cm of wa-
ter, measured in the recumbent position,
are considered abnormal in children in
the appropriate clinical context.18
Treatment of IIH in the acute phase
consists of acetazolamide, a carbonic
anhydrase inhibitor that reduces CSF
production. Common side effects of
acetazolamide include paresthesia and
altered taste. Other medications that
have been utilized in IIH are topiramate
and furosemide. If the child presents
with acute loss of vision (not transient
obscurations), immediate referral to a
neurosurgeon is critical. Surgical op-
tions to reduce CSF pressure may be
undertaken to restore vision. The choice
of procedure may vary depending on the
expertise of the surgeon. Lumbo-perito-
neal shunts are avoided in many institu-
tions due to potential risks of infection
PEDIATRIC ANNALS • Vol. 47, No. 2, 2018
SPECIAL ISSUE ARTICLE
Figure 3. Magnetic resonance venogram of the
brain in a child with idiopathic intracranial hy-
pertension demonstrating stenosis of the right
transverse sinus.
and need for revisions in the future,
although evidence-based guidelines
regarding which intervention is most
beneficial are lacking. In the long term,
weight loss should be encouraged. Re-
peated lumbar puncture or draining of
large volumes of CSF is not usually
beneficial.
Neoplasms of the Central Nervous
System
Brain tumors are the second most
prevalent cancer in childhood. The
incidence is 5 to 6.22 per 100,000 in
children age 0 to 14 years. Most chil-
dren younger than age 14 years with
brain tumors have low grade gliomas.19
Nearly one-half of all brain tumors of
childhood arise below the tentorium
(ie, close to the cerebellum and brain
stem) and therefore present with rela-
tively easily recognizable signs and
symptoms20 (Figure 4).
Headache tends to be the first pre-
senting symptom of intracranial tumors,
and when accompanied by certain red
flags, appropriate imaging is manda-
tory. The cardinal associated symp-
toms include a headache that awakens a
child at night from sleep, early morning
Figure 4. Magnetic resonance image of the brain.
Axial images with contrast, showing subependy-
mal giant cell astrocytoma in a child with tuber-
ous sclerosis impinging on the foramen of Munro.
headaches, posterior/occipital location
of pain, projectile emesis, new onset of
lethargy or somnolence, and diplopia.
Photophobia and phonophobia, as seen
with migraines, are unusual with space-
occupying brain lesions. Nausea is often
seen in children who have tumors that ei-
ther directly or indirectly (through ede-
ma) impinge on the floor of the fourth
ventricle where the nausea center, or
area postrema, is located. Focal deficits
such as cranial nerve involvement, espe-
cially restriction of upward gaze, hemi-
paresis, or ataxia, are signs suggestive
of intracranial pathology. An abducens
nerve palsy with failure of abduction of
the eye is an important physical finding
that raises suspicion for increased intra-
cranial pressure. Papilledema is noted in
only 41% of children with hydrocepha-
lus; therefore, overreliance on this phys-
ical finding should be avoided.21
Importantly, response to treatment
with nonsteroidal anti-inflammatory
drugs does not exclude a secondary
cause for headaches. About 44% of pa-
tients with secondary headache report
significant reduction of symptoms from
nonsteroidal anti-inflammatory drugs
and antiemetics.22
e77

Figure 5. Magnetic resonance image of brain in a
sagittal view demonstrating astrocytoma poste-
rior to the cerebellum.
In addition to a detailed and direct-
ed history and a bedside neurological
evaluation, a thorough general physical
examination can lead an astute physi-
cian to look for certain brain tumors.
For instance, hypomelanotic macules,
facial angiofibroma, ungual fibromas,
or shagreen patches are findings that
suggest tuberous sclerosis complex
(TSC). These patients may develop a
subependymal giant cell astrocytoma
(SEGA). SEGA is observed in 5% to
15% of patients with TSC, and can
produce clinical symptoms secondary
to obstructive hydrocephalus, which
include headache, emesis, and cranial
neuropathies23 (Figure 5).
A computed tomography scan is
indicated in situations in which the
child has altered mental status, focal
prolonged seizures, and if there are
contraindications to MRI. The radia-
tion exposure to the patient, however,
limits its use. MRI of the brain, with
and without contrast, is the gold stan-
dard to evaluate and diagnose a brain
tumor. However, there is some evidence
that repeated exposure to gadolinium in
pediatric patients leads to deposition of
contrast in the brain parenchyma.24 The
possible long-term neurotoxic effects
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SPECIAL ISSUE ARTICLE
Figure 6. Magnetic resonance axial images of the
brain in a child with brain abscess and marked
surrounding edema (arrow).
of contrast deposition have not yet been
clearly elucidated.25
Treatment of tumors is based on
histology. Most require neurosurgical
intervention. Low-grade tumors may
only require curative resection whereas
atypical or high-grade tumors such as
medulloblastomas, require resection,
chemotherapy, and/or radiation thera-
py. Secondary headaches are often dif-
ficult to treat in the setting of a brain
tumor. Decreasing the edema with
steroids can be a helpful preliminary
measure.
Infection
Infectious etiologies of the central
nervous system should be considered
when evaluating a patient with acute
onset headache in the setting of sys-
temic illness, fever, and/ or altered
mental status.
Bacterial and viral meningitis can
present as severe holocephalic head-
ache with associated photophobia and
neck stiffness. In addition, physical
examination findings such as tachy-
Figure 7. Chiari I malformation with herniation of
the tonsils of the cerebellum (upper arrow) and
associated syrinx in the spinal cord (lower arrow).
Magnetic resonance image of the brain and spine
sagittal view T2-weighted images.
cardia and hypotension, a generalized
rash, and irritation when neck is flexed
or legs are raised (ie, Brudzinski and
Kernig signs, respectively) may en-
courage the clinician to pursue testing
for meningitis. Encephalitis refers to
inflammation of the brain parenchyma
and presents with symptoms sugges-
tive of meningitis, including holoce-
phalic headache, but with additional
abnormalities in brain function such
as altered mental status (drowsiness,
irritability), motor or sensory deficits,
altered behavior and personality, and
seizures. Encephalitis can be caused by
viral, bacterial, and autoimmune con-
ditions.
Autoimmune encephalitis is a new-
ly described entity with a progressive
clinical course. The hallmark features
include headache in association with a
constellation of psychiatric and behav-
ioral symptoms such as hallucinations
and psychosis, seizures, memory dys-
function with short-term memory loss,
speech disorders, and decrease in level
of consciousness.26 A conversion disor-
Copyright © SLACK Incorporated

der is often suspected in such children;
therefore, knowledge of this entity is
crucial to clinicians.
Brain abscess, although rare, should
be considered in the differential diagno-
sis of a child with localized headache,
focal neurological signs, and a recent
history of an infection in the central
nervous system or other organ systems
such as the heart or blood. Clinical situ-
ations associated with intracranial ab-
scess include otitis media, mastoiditis,
infective endocarditis, and patients who
are immune compromised. The edema
surrounding an abscess may lead to a
headache that is very difficult to treat.
Children with a brain abscess also pres-
ent with symptoms and signs of raised
intracranial pressure such as vomiting,
papilledema, and abducens palsy, as
noted earlier in this article.
MRI findings may aid in the differ-
ential diagnosis of an infectious etiol-
ogy for headache. Herpes encephalitis,
for instance, has a propensity to in-
volve the temporal lobes and the frontal
lobes.27 MRI of a brain abscess reveals
a “ring enhancing” lesion (Figure 6),
whereas in autoimmune encephalitis
imaging abnormalities may not be pres-
ent in more than 50% of children.26
Treatment is based on etiology.
When an infection of the central ner-
vous system is suspected, the child
should be admitted for immediate ini-
tiation of antibiotics or antiviral therapy
(acyclovir for herpes simplex encepha-
litis), supportive treatment such as pain
management, neurological evaluation,
and close supervision. Imaging, spinal
fluid analysis, and other laboratory in-
vestigations should not take precedence
over initiation of treatment and stabili-
zation of airway and circulation.
Chiari Malformation
Structural causes other than a tumor
can lead to persistent headaches that are
PEDIATRIC ANNALS • Vol. 47, No. 2, 2018
SPECIAL ISSUE ARTICLE
often refractory to medical treatment.
Chiari I malformation is one such ex-
ample. Chiari malformation refers to
the downward displacement of the cere-
bellar tonsils (the inferior-most portion
of the cerebellum) into the foramen of
magnum24 (Figure 7). The prevalence
of Chiari I malformation is estimated to
be 0.6% to 0.9% in the general popula-
tion and about 1% in children.28 Chiari
malformation is most often found in-
cidentally when imaging is performed
for headache, but it rarely causes symp-
toms unless the displacement of the
tonsil is more than 5 mm in relation to
the foramen magnum. Certain systemic
diseases such as Ehlers-Danlos syn-
drome may predispose a child to Chiari
lesions.29
Pain is present in 60% to 70% of
affected children.30 These headaches
have a characteristic feature of wors-
ening upon neck extension or with
Valsalva maneuvers such as coughing
or sneezing. They may also be associ-
ated with scoliosis. Young children may
show signs of failure to thrive and/or
brainstem dysfunction such as respirato-
ry irregularities, dysphagia, cough, and
tongue atrophy, in addition to headache.
Cerebellar signs such as truncal and
limb ataxia may also be seen. Children
older than age 3 years often present with
neck pain, occipital headache, vertigo,
sensory changes such as numbness or
pins and needles sensation in their arms
and legs, ataxia, syncope, sleep apnea,
and drop attack.31
When Chiari malformation is sus-
pected in a child, an MRI of the brain
with and without contrast should be per-
formed. Contrast material will help rule
out a parenchymal mass that can also
cause similar symptoms. If Chiari I mal-
formation is found, the complete spine
should be imaged with an MRI with and
without contrast to identify a syrinx (a
fluid-filled cavity that forms within the
spinal cord). A Chiari malformation that
is symptomatic requires neurosurgical
referral and intervention. Symptoms can
recur and patients may need a second
decompression. In a series of 256 chil-
dren with Chiari I malformation, 22%
had symptom recurrence and 7% of pa-
tients required repeat decompression.32
The patients that benefit the most from
a surgical procedure are those who are
carefully chosen based on herniation
size, anatomy, and clinical symptoms.
CONCLUSION
Headaches that may have serious
neurological consequences can be dis-
tinguished from primary headache dis-
orders by bedside clinical testing and
conclusively diagnosed by appropriate
brain imaging. Knowledge of symptoms,
by brain imaging in most instances, and
physical signs that are suggestive of se-
rious intracranial pathology can enable
the pediatrician to identify diseases that
require immediate intervention, whether
it be medical or surgical.
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